[CIS PIDD] [cis-pidd] 20 year old female with low IgA and autoimmune gastritis

Fri Feb 13 14:34:15 EST 2015

Hello Kim,
Hope you are doing well.

Could you try Entocort – small bowel protocol?
Entocort enteric coated 3mg—
•       1 capsule whole
•       1 capsule opened on food
•       1 capsule chewed and swallow

I have a few adult CVID patients and 2 with enteropathy post RTX who have responded to this protocol. These patients not only had gastritis , but also enteritis /colitis, hence this regimen.
http://www.ncbi.nlm.nih.gov/pubmed/22810979

I am not sure if you need to dose it this way if you only have gastritis to deal with, just the chewed and swallow should suffice. The patient  should open the first capsule, place the granules in applesauce, and chew before swallowing, typically in the morning. She should avoid CYP3A4 inhibitors ( drugs, fruits like grapefruit, etc.) concurrently.

Best,
Avni

Avni Joshi, MD, MSc
Mayo Clinic
Rochester, MN 55905

From: Soheil Chegini [mailto:schegini at yahoo.com]
Sent: Friday, February 13, 2015 11:33 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] 20 year old female with low IgA and autoimmune gastritis

Hi Kim,

I have no answer to the question you are asking, but instead maybe a suggestion for an alternative to immunosuppressive treatment.

You did not specify what exactly her symptoms were, but I wonder whether she might respond to octreotide. Unfortunately, my experience with this condition is from my fellowship days when I worked on an animal model of organ-specific autoimmunity in mice after neonatal thymectomy. I have not had cared for any patients with this diagnosis, but have a patient who is on octreotide for intestinal lymphangiectasia, and he has done substantially better on it.

Best wishes,
Soheil

Soheil Chegini, M.D.
Exton Allergy & Asthma Associates
656 West Lincoln Hwy.
Exton, PA 19341
Phone: (610) 269-3066
Fax: (610) 269-8615

________________________________
From: "Risma, Kimberly" <Kimberly.Risma at cchmc.org<mailto:Kimberly.Risma at cchmc.org>>
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Friday, February 13, 2015 10:31 AM
Subject: [cis-pidd] 20 year old female with low IgA and autoimmune gastritis

Hello colleagues,

I have followed a young lady for a few years who presented for immune evaluation at 19 years of age with debilitating abdominal distress secondary to documented active autoimmune gastritis.
She had elevated gastrin levels, (+) antiparietal Antibodies, and anti thyroperoxidase antibodies suggesting  a variant of autoimmune polyendocrinopathy.
Her symptomatology responded to oral steroid therapy and she was transitioned successfully to sirolimus as she did not tolerate a “no treatment” approach.
Over the last 3 years of therapy her gastrin levels have fallen nicely although her antiparietal Ab remains strongly (+).
She becomes symptomatic again when she stops sirolimus for a week. Her immune evaluation was essentially normal except for a mildly decreased IgA.
She has no history of recurrent infections. She reportedly developed Herpes Zoster once on sirolimus, but it resolved successfully without antivirals.

My question for the group-
She is wanting to have a baby and would like to stop her medications to do so (I don’t have any experience with pregnancy and sirolimus).
Are there any other options other than trying to find a low dose steroid approach?

Thanks in advance, Kim

Kimberly Risma MD PhD
Assistant Professor
Director, Allergy/Immunology Fellowship
Division of Allergy/Immunology
Cincinnati Children’s Hospital Medical Center

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P: +1.414.224.8095
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